Intractable Rare Dis Res. 2017;6(1):72-75. (DOI: 10.5582/irdr.2016.01081)

Audiological findings from an adult with thin cochlear nerves.

Prabhu P, Shivaswamy J

Reported here are audiological findings from an adult with thin cochlear nerves. Magnetic resonance imaging (MRI) revealed that he had a thinner cochlear nerve in the left ear than in the right ear. He had a higher degree of hearing loss in the left ear and poor speech recognition scores for both ears. He had normal middle ear and cochlear functioning. The auditory brainstem response and acoustic reflexes were absent, indicating a retrocochlear pathology. Long latency responses (LLR) revealed normal cortical functioning. Hence, implantation of an auditory brainstem implant might be an option, but the patient would need to be aware of its limitations. This case highlights the importance of MRI in evaluating congenital malformations of the cochlear nerve when audiological findings indicate a retrocochlear pathology.

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